DOENÇA DE WOLMAN: UMA PATOLOGIA RARA

Abstract

Wolman disease or Wolman xanthomatosis is a severe childhood form of hereditary lysosomal lipid storage disorders secondary to acid lipase (sterol esterase) deficiency. It is an ultra-rare condition with an incidence of 1/500,000 cases. In this disease, the rarest form of acid lipase deficiency, these lipids accumulate in most tissues.  It is an allelic variant of cholesterol ester storage disease. It has autosomal recessive inheritance.